adrenal diseases Flashcards
what is adrenal insufficiency
inadequate adrenocortical function
what are the types of adrenal insufficiency
primary (acute and chronic)
secondary
what causes secondary adrenal insufficiency, what is most common
anything outwith the adrenal gland:
lack of ACTH stimulation
iatrogenic = most common
pituitary/hypothalamic disorder
give an example of an iatrogenic cause of adrenal insufficiency
excess exogenous steroids due to medication
what are the cause of chronic primary adrenal insufficiency, what is most common
addison's disease = most common congenital adrenal hyperplasia adrenal TB (rare in uk) adrenal malignancy (rare)
what are the investigations of adrenal insufficiency
CT adrenals
what are the causes of acute primary adrenal insufficiency
rapid withdrawal of steroid treatment
massive adrenal haemorrhage
adrenal crisis
what is Addison’s disease
type of adrenal hypofunction
what causes Addison’s disease
autoimmune destruction of the adrenal cortex
what are the symptoms of Addison’s disease, describe their onset
anorexia fatigue/weakness dizziness abdominal pain vomiting and diarrhoea skin pigmentation = buzzword
why does skin pigmentation happen in Addison’s disease
high levels of ACTH cross-react with melanocyte receptors
what does decreased mineralocorticoids cause in Addison’s disease
hyperkalaemia
hypotension/postural hypotension
what does decreased glucocorticoids cause in Addison’s disease
hyopglycaemia
what investigations should you do in what does in Addison’s disease
Na and K levels
ACTH levels
short Synacthen test
adrenal antibodies
what level of ACTH would you expect in Addison’s disease
HIGH
what levels of Na and K would you expect in Addison’s disease
low Na
high K
what should make you suspect Addison’s disease until proven otherwise
low Na
high K
when would you treat Addison’s disease
as soon as its suspected, don’t wait for diagnosis to be confirmed
what is the treatment of Addison’s disease
hydrocortisone
fludrocortisone
what are the sick day rules surrounding hydrocortisone treatment in Addison’s disease
Temp >38 = hydrocortisone dose doubled
Temp >39 = hydrocortisone dose tripled
when would hydrocortisone be given IV in Addison’s disease
if patient is systemically unwell
what must be done when giving fludrocortisone
monitor BP
monitor K
what is the treatment of adrenal crisis
ASAP:
IV saline
IV hydrocortisone
what is adrenal crisis
severe adrenal insufficiency
what is congenital adrenal hyperplasia
rare autosomal recessive condition associated with enzyme defects in the steroid pathway
what are the two types of congenital adrenal hyperplasia
classical
non-classical
what are the investigations of congenital adrenal hyperplasia
genetic mutation analysis
basal or stimulated 17-OH progesterone
what is the treatment of congenital adrenal hyperplasia in adults
control androgen excess
restore fertility
avoid steroid over-replacement
what is the treatment of congenital adrenal hyperplasia in children
glucocorticoid replacement = suppresses ACTH
mineralocorticoid replacement in some
surgical correction of genitals
what are the symptoms of classical congenital adrenal hyperplasia
female = genital ambiguity male = scrotal discolouration
poor weight gain
adrenal insufficiency (around 2-3 weeks)
biochemical pattern similar to Addison’s
what are the symptoms of non-classical congenital adrenal hyperplasia
Hirsute Acne Oligomenorrhoea Precocious puberty Infertility or sub-fertility
what symptoms would you expect in a patient lacking in CRH or ACTH
similar to addison’s but will have pale skin and intact aldosterone production
what is the treatment of a lack or CRH /ACTH
hydrocortisone replacement
what is cushing’s syndrome e
excess of cortisol
how is mainly affected by cushings
women aged 20-40
what causes cushings syndrome
pituitary adenomas adrenal adenomas ectopic ACTH production e.g. lung cancer alcohol steroid medication
what is excess cortisol caused by a pituitary adenoma known as
cushings disease
what is the difference between cushings syndrome and cushings disease
syndrome = caused by anything resulting in high cortisol levels disease = caused by high cortisol levels due to a pituitary adenoma
what are the symptoms of cushings syndrome
proximal myopathy/wasting osteoporosis thin skin = easy bruising & striae diabetes central obesity high BP virilism hirsuitism acne olgio/amenorrhoea
what is the investigations of cushings syndrome
high dose oral exogenous steroid (dexamethasone) low dose dexamethasone overnight dexamethasone suppression test 24hr urine collection diurnal cortisol variation
what is the gold standard investigation for cushings, what results would you expect
low dose DST
cortisol <50 = not cushings
cortisol >130 = defo cushings
what is the treatment for cushings caused by a pituitary issue
Hypophysectomy and
external radiotherapy if recurs
Bilateral adrenalectomy
what is the treatment for cushings caused by an adrenal issue
adrenalectomy
what is the treatment for cushings caused by an ectopic issue
remove source
bilateral adrenalectomy
what drugs can be used to treat cushings
Metyrapone
Ketoconazole
Pasireotide LAR = somatostatin analogue
when would you prescribe drugs in cushings
if all else fails or while waiting for radiotherapy to work
what is primary aldosteronism
autonomous production of aldosterone independent of its regulators
what are the regulators of aldosterone
angiotensin II
K+
what are the causes of primary aldosteronism, what is most common
adrenal adenoma
bilateral adrenal hyperplasia = most common
unilateral hyperplasia
genetic mutations
what is primary aldosteronism caused by adrenal adenoma called
Conn’s syndrome
what are the symptoms of primary aldosteronism
significant hypotension
hypokalaemia
alkalosis
what are the investigations of primary aldosteronism
ARR-aldosterone to renin ratio, if raised do saline suppression test to confirm PA
adrenal CT
adrenal vein sampling
what is the treatment of primary aldosteronism caused by an adrenal adenoma (conn’s)
surgery = unilateral laparoscopic adrenalectomy
what is the treatment of primary aldosteronism caused by bilateral hyperplasia
MR antagonist = spironolactone or eplereone
describe an adrenocortical adenoma
well circumscribed, encapsulated lesion composed of cells resembling adrenocortical cells
yellow surface
describe an adrenocortical carcinoma
large
frequent, abnormal mitoses
lack of clear cells
capsular or vascular invasion
what is phaeochromocytoma, where does it originate
neuroendocrine tumour of the medulla of the adrenal gland, originating in the chromaffin cells
what does phaeochromocytoma secrete
catecholamines
what is a phaeochromocytoma located outside of the adrenal called
paraganglioma
why is a phaeochromocytomadark brown in colour
potassium dichromate causes oxidation of catecholamines within tumour
what is the classical triad of symptoms of phaeochromocytoma
hypertension
headache
sweating
what are the symptoms of phaeochromocytoma
hypertension headache sweating palpitations SOB consitpation anxiety/fear weight loss
what is phaeochromocytoma associated with
MEN2
Von-hippel-lindau syndrome
Neurofibromatosis
what are the two purposes of the investigations of phaeochromocytoma
confirm catecholamine excess
identify source of excess catecholamines
what investigations confirm excess catecholamines in phaeochromocytoma
2 x 24hr catecholamines or metanephrins in urine
catecholamines in plasma
what are the issues in trying to identify catecholamine excess
they are naturally raised in HF
catecholamine secretion is episodic
what investigations identify source of raised catecholamines in phaeochromocytoma
MIR
MIBG
PET scan
what is the treatment of phaeochromocytoma
alpha blocker THEN add beta blocker
surgery
chemo if malignant
what alpha blocker is used to treat phaeochromocytoma
phenoxybenzamine
what beta blocked can be used to treat phaeochromocytoma
propanolo, atenolol, metoprolol
what is a neuroblastoma
rare tumours composed of primitive appearing cells which can show maturation and differentiation towards ganglion cells.
where do neuroblastoma arise
40% adrenal medulla
the rest are mainly along the sympathetic chains
